Idiopathic throbocytopenic purpura(ITP)
.Purpura is local hge. to the skin.
.Etiology by:- 1-Increase capillary fragility duo to drugs…. steroids 2-Thrombocytopathies by aspirin tablets 3-Thrombocytopnia: A- platelets production decrease as in aplastic anemia,
cytotoxic drugs B-increase platelets consumption as in IVC in sepsis, large haemangiomas C-Increase platelets destruction as in autoimmune disease
SLE, infection as IMN, ITP, drug reaction as quinine D-Increase splenic sequestration of platelets as any
condition causing splenomegaly as portal hypertention
Clinical features:-
.skin and mucus membrane purpura
.epistaxis
.menorrahgia
.simple wound bleeding
.urinary, GIT, haemarthrosis rare
.intracranial he
.Torniquet test +ve
.25% has palpable spleen
.Huge splenomegaly suggest the diagnosis is not ITP.
Investigations:-
1- increase bleeding time ( clotting and prothrombing time is normal)2-platelet count < 60 0003-bone marrow biopsy shows plentiful supply of megakaryocytes
Treatment:-
Depend on behavior of the disease and age of the patient but no splenectomy during acute attaches of ITP.
.children 75% regress by cortisone and azothiaprine
.splenectomy in sever cases & girls approaching menarche
.adult and sever if ITP persist > 6-9 months do splenectomy
. By splenectomy
60% cured, 20% improved, 15% no benefit
Erythrocytes disorders( haemolytic anemia):-
Cases amenable to splenectomy:-
1-Hereditary spherocytosis2-Acquired autoimmune H. anemia3-Thalassemia4-Hereditary elliptocytosis5-Pyruvate kinase defeciency
1- Herditary sphyrocytosis
-duo to dysfunction or deficiency in one of the RBC membrane proteins which increase permeability to Na.
-Mendelian autosomal dominant-male=female-increase RBC permeability to Na so more fragile.
Clinical features:-
-mild jaundice, anemia, splenomegaly-pale, lassitude and undue fatigue-RBC crisis sometimes occur , precipitated by acute
infection even may cause death( pyrexia, abdominal pain, N,V, extreme pallor and jaundice)
-in adult may develop jaundice, attacks of biliary colic
On examination .spleen enlargement palpable, sometimes liver also palpable.chronic leg ulcer in adults
Investigations:-1- Fr agility test ( N is 0.47% NaCl haemolysis occur but
it is 0.60% or even more )2-Reticular count3-Faecal urobilinogen is high4-Radioactive chromium 515- sonography to show gal stones
Treatment:-Splenectomy -at about 7 - years of age to decrease gal stone
formation and chance for infections
2-Acquired haemolytic anemia:-
Duo to complement or Ig on RBC which triggered by macrophages Fc receptors in spleen( culling effect),caused by:-
-drugs as methyl dopa-disease like SLE -unknown causes
Clinical features :- -common> 50 years of age, more in female has mild
jaundice and anemia-spleen enlargement 50%-gal stones 20%
Investigation:--Coombs test is positive
Treatment:-
-no treatment-acute self limiting -treatment with cortisone-splenectomy if:- 1-corticosteroid ineffective 2-patient had complications from using cortisone 3-cortisone contraindicated as in PU disease
80% respond to splenectomy
3-Hereditary elliptocytosis:-
When the erythrocytes circulate it elongated so less sequestrated.
4-Thalassemia (Mediterranean anemia, Coolys anemia)-duo to Hb synthesis defect-dominant trait-alfa ,beta, gamma type ,but mostly is beta chain result
in Hb A decrease and has intracellular ( Heinz bodies) so premature RBC destruction occur
-from hetrozygous minor to …… homozygouse major
Clinical features:-
-chronic anemia, jaundice, splenomegaly
-Major in children cause retarded growth, enlarged head, slantinge eyes, depressed nose, leg ulcer, jaundice and abdominal distention
Investigations:-
1- small RBC ,resist osmolysis2-nucleated RBC 3- Hb electrophoresis
Treatment:--blood transfusion-splenectomy by: .repeated blood transfusion .uncomfortable and painful
splenomegaly
5-Sickle cell anemia:-
-is heridetary haemolytic anemia
-autosomal codominanat
-common in Africans origin in which normal Hb A replaced by Hb S
-As Hb S crystallized when O2 tension decrease so elongate, increase blood viscosity and obstruct the flow in open and closed system of the spleen circulation.
-cause painful intermittent episodes of abdominal pain which may result in auto -splenectomy
Clinical features:-
-9% Africans origin
-mostly asymptomatic
-symptoms depend on which organ is involved like; joint pain, priapism, neurological abnormality, skin
ulcers, abdominal pain due to intestinal bowel ischemia.
Investigations:--blood film show sickle shapped cells-Hb electrophoresis
Treatment:--avoid hypoxia specially during general anathesia
-adequate hydration
-Splenectomy may be helpful
-in streptococcus infection may precipitate the acute attacks so treatment is by giving RBC and splenectomy
Schistosomiasis:-
-75%is sch.mansoni, 25% is sch. Haematobium-common in Africa-Splenic hyperplasia occur due to phagocytosis of the
disintegrated worms, ova, toxins and liver fibrosis which may cause portal hypertension.
Clinical features :--male>female-splenomegaly features
Investigations:-
-urine and stool for ova-Liver function test-Hb decrease
Treatment:--medical treatment-splenectomy if spleen is bulky and painful
Tuberculosis:-
-not uncommon-age 20-40 years
clinical features:--asthenia, lose of weight, evening fever + splenomegaly-may produce portal hypertension-cause cold abcessInvestigation:--culture and guinea- pig inoculation
Treatment:-- Anti-tuberculose drugs + splenectomy if there is no
active disease in the body .
Gauchers disease:-
-storage of abnormal lipioid( glucocerebroside) in reticuloendothelial cells
-common in Jewish &Slavonic races-rare disease-spleen may reach 4-5 kg.-appear < 12 years of age so in early childhood-bone pain ,pathological #, jaundice, anemia, yellow –brunish
discoloration of the skin of hands& faces-conjunctiva thickening (pinguecula)-early satiety and abdominal distension due to splenomegaly-diagnosis by Gaucher cells in bone marrow
Treatment :--by splenectomy may be helpful -partial splenectomy in children
Amyloidosis:-
-abnormal extracellular protein deposition
-multiple organs involved
-clinically may be asymptomatic or patient come with MOF ( multiple organ failure)
-if associated with splenomegaly do splenectomy to relief symptoms of splenomegaly
Sarcoidosis:-
-inflammatory disease characterized by non-caseating granulomatouse lesions affecting the tissue.
-common in young adults
-Involve mostly lung
-sings & symptoms are nonspecific as malase, fatigue
-25% has splenomegaly, and spontaneous rapture of spleen may occur
-treatment is by splenectomy when sings and symptoms are present.
Feltys Syndroom:-
-duo to immune complex coated the surface of the leucocytes
-is chronic arthritis + leucopenia < 2000c/dl + splenomegaly
-if light arthritis associated with the leucopinea and splenomegaly called primary splenic neutropenia.
-treatment by giving corticosteroids, methotrexate-splenectomy may be helpful.
Neoplasms:-
-haemangiomas , sometimes haemangiosarcoma treated by splenectomy
-lymphoma is the common one treatment by splenectomy
Hypersplenism duo to portal hypertention:-
-cause decrease of platelets & granulocytopenia
-Treatment by splenectomy + shunt
Tropical splenomegaly:-
-especially in children occur
-duo to malaria, kalazar, schistosomiasis, malnutrition
-abnormal response to malaria parasite and unusual spices of plasmodia
-huge splenomegaly 2-4 kg.
-anemia+ thrombocytopenia
-treatment by splenectomy, but in endemic area with malaria give antimalarial drugs ( proguanil)
Splenectomy:-
Indications:-1-Trauma-blunt or penetrating2-Iatrogenic. e.g. mobilization of splenic flexture in
operation for ca rectum.3-As part of other operations eg. Carcinoma of the
stomach.4-Staging of lymphoma5-Erythrocytes disorders as hereditary
spherocytosis,acquired haemolytic anemia, auto immune haemolytic anemia( Mediterranean anemia)…etc
6-Haemoglobinopathies as thalassemia, sickle cell anemia, porphyria…..etc
7-Leukocytes disorders as Feltys disease….etc8-Platelets disorders as ITP….etc
Splenectomy indications cont.9-Bone marrow disorders as myeloproliferative disease10-Cysts parasitic or nonparasitic.11-Neoplastic as angioma, primary fibrosarcoma12-Infections as : . bacterial…..TB . protozoal & parasites ….schisotomiasis, tropical
splenomegaly13-Abcess14-Storage & infiltrative disease as : .Gaucher s disease, sarcoidosis, amyloidosis15-Miscellanouse disorders as: .portal hypertension, splenic vein thrombosis .
Splenectomy cont.
Preoperative preparation for elective splenectomy:-
1-7-10 days before operation give vaccination for
pneumococcal….. 2-Hb more than 10 mg/dl
3-Prepair 2-4 unit blood for huge splenomegaly cases
4-Treat thrombocytopenia
5-If patient receiving oral cortisone , shift to cortisone schedule by giving intravenouse cortisone.
Post operative complications:-
1- Hemorrhage
2-Gastric dilatation
3-Haematemesis
4-Left basal atelectasis + pleural effusion, pneumonia
5-Left subphrenic abscess
6-Damage to tail of pancrease may cause local pancreatic abscess, pancreatic fistula, pancreatitis
Postoperative complications cont.
7-Wound infection
8-Thrombo-embolic phenomena causing deep vein thrombosis, portal vein thrombosis
9-Gastric fistula
10-Postsplenectomy septicemia by overwhelming postsplenectomy infection ( OPSI) by streptococcus pneumonia, nisseria meningitides, haemophilus influenzae, salmonella, bacteroids
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